Abstract

Pulmonary Langerhans' cell histiocytosis (histiocytosis X) is an uncommon, diffuse interstitial lung disease of unknown cause, mostly presenting in young smokers. Association of pulmonary Langerhans' cell histiocytosis with a malignant neoplasm is rare. We present and discuss the case of a 48-year-old man (ex-smoker) with metastasising malignant melanoma. A few months after chemotherapy and a modified Whipple procedure for retroduodenal metastasis of a malignant melanoma, computer tomographic scans revealed intrapulmonary "ring-shaped structures". Endobronchial biopsies and bronchioalveolar lavage showed no evidence of neoplasm or inflammation. Open-lung biopsy was performed and revealed pulmonary Langerhans' cell histiocytosis. To our knowledge this is the first reported case of pulmonary Langerhans' cell histiocytosis in association with malignant melanoma. Chemotherapy for malignant melanoma may be related to the development of pulmonary Langerhans' cell histiocytosis.

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